RESUMEN
BACKGROUND: Fox-Fordyce (FFD), also known as apocrine military, is an uncommon chronic inflammation of the apocrine sweat glands. It is characterized by pruritic, papular eruptions in apocrine-gland-bearing regions. FFD was described a century ago, but the exact pathogenesis of the disease and the management are not well understood. AIMS: This paper provides a wide understanding of the pathophysiology, clinical findings, and management of Fox-Fordyce disease. Its aim is to help the physician to diagnose and manage this entity accordingly. METHODS: A research was done using PubMed database on 12 April 12, 2020, and in order to retrieve all case reports, case series, cohort studies, randomized, and nonrandomized clinical trials were included describing FFD among patients. RESULTS: A total of 43 articles and 68 patients were included in the study. The majority of patients were young females. The disease was bilateral in 90%, affected the axillae and to a lesser extent the pubic and the periareolar areas and rarely the thoracic area, the abdominal area, and the face. FFD followed a relapsing and remitting course, and an evident improvement in disease course was noted after menopause. CONCLUSION: The typical FFD patient is a post-pubertal female and pre-menopause, presenting with pruritic papules in apocrine-gland-bearing regions. FFD can be sporadic or occurs in family, and it can be asymptomatic in 1/(3-4) of patients and can be triggered by laser hair removal and hormonal changes. Further randomized clinical trials assessing different treatment of FFD are now warranted.
Asunto(s)
Enfermedad de Fox-Fordyce , Remoción del Cabello , Glándulas Apocrinas , Axila , Epidermis , Femenino , Enfermedad de Fox-Fordyce/diagnóstico , Enfermedad de Fox-Fordyce/terapia , HumanosRESUMEN
Fox-Fordyce disease (FFD) is a rare pruritic dermatosis whose etiology has not been fully explored. It is mostly seen in women and presents as pruritic follicular papules at the apocrine (gland-bearing) regions, including the axilla, groins, perineum, and areola mammae, as well as the umbilicus. Treatment for FFD is extremely challenging in that there is no curative treatment for it. We report the case of a 26-year-old woman who was refractory to many treatments but who responded to calcipotriol betamethasone with rapid remission of her symptoms.
Asunto(s)
Enfermedad de Fox-Fordyce , Remoción del Cabello , Adulto , Axila , Betametasona/uso terapéutico , Calcitriol/análogos & derivados , Femenino , Enfermedad de Fox-Fordyce/diagnóstico , Enfermedad de Fox-Fordyce/tratamiento farmacológico , HumanosRESUMEN
Fox-Fordyce disease (FFD), also known as apocrine miliaria, is a rare and chronic skin disease characterized by itching and skin-colored, light brown or yellowish papules. FFD typically affects postpubertal young women between 13 and 35 years. The etiology is not completely known, but a hormonal component is in discussion. Furthermore, exacerbating factors like laser hair removal and hyperhidrosis have been described. Treatment of FFD is quite challenging, as the reported modalities mostly show limited success.
Asunto(s)
Enfermedad de Fox-Fordyce , Hiperhidrosis , Axila , Femenino , Enfermedad de Fox-Fordyce/diagnóstico , Enfermedad de Fox-Fordyce/terapia , Remoción del Cabello , Humanos , Hiperhidrosis/diagnóstico , Hiperhidrosis/terapia , Piel , Adulto JovenRESUMEN
Fox-Fordyce disease (FFD) is a rare chronic disorder characterised by persistent inflammation because of the obstruction of apocrine sweat glands, which is a key factor of pathogenesis. The treatment of FFD is known to be difficult, and the modalities of treatment have not yet been widely studied. We report the successful treatment of a case of bilateral areolar FFD by a combination of surgical excision and 1550-nm fractionated erbium glass laser in an 18-year-old woman. The patient presented with a bilateral areolar eruption of multiple, severely pruritic, 3-4 mm skin- to grey-coloured folliculocentric dome-shaped papules. The initial treatment plan was for bilateral surgical excision of the larger and more highly elevated papules via circumferential dermal excision, which was intended to maintain the areolar contour and minimise distortion. A 1550-nm fractional erbium glass laser was then used to control the remnant lesions. The patient was recurrence-free at 14 months after the final laser treatment, and she was fully satisfied with the treatment results.
Asunto(s)
Enfermedad de Fox-Fordyce/diagnóstico , Enfermedad de Fox-Fordyce/cirugía , Terapia por Láser/instrumentación , Pezones/cirugía , Adolescente , Erbio , Femenino , Vidrio , Humanos , Resultado del TratamientoRESUMEN
No disponible
Asunto(s)
Humanos , Masculino , Adulto Joven , Enfermedad de Fox-Fordyce/complicaciones , Enfermedad de Fox-Fordyce/diagnóstico , Enfermedades del Pene/diagnóstico , Crioterapia , Terapia por Láser/métodos , Enfermedad de Fox-Fordyce/fisiopatología , Enfermedad de Fox-Fordyce/terapia , Pene/microbiología , Pene/patología , Anamnesis/métodos , Anamnesis/normas , Estudios RetrospectivosRESUMEN
No disponible
Asunto(s)
Humanos , Femenino , Adulto Joven , Axila/lesiones , Enfermedad de Fox-Fordyce/diagnóstico , Terapia por Láser/efectos adversos , Remoción del Cabello/efectos adversos , Tretinoina/uso terapéuticoAsunto(s)
Angiofibroma/patología , Neoplasias Faciales/patología , Inhibidores de Proteínas Quinasas/uso terapéutico , Esclerosis Tuberosa/diagnóstico , Erupciones Acneiformes/diagnóstico , Erupciones Acneiformes/etiología , Acrodermatitis/diagnóstico , Acrodermatitis/etiología , Asteraceae/efectos adversos , Dermatitis Alérgica por Contacto/inmunología , Enfermedad de Fabry/diagnóstico , Dermatosis Facial/patología , Enfermedad de Fox-Fordyce/diagnóstico , Enfermedad de Fox-Fordyce/tratamiento farmacológico , Infecciones por VIH/complicaciones , Humanos , Interferones/uso terapéutico , Enfermedades Cutáneas Vesiculoampollosas/etiología , Serina-Treonina Quinasas TOR/antagonistas & inhibidores , Terminología como Asunto , Esclerosis Tuberosa/tratamiento farmacológico , Esclerosis Tuberosa/genética , Esclerosis Tuberosa/patología , Urticaria/etiología , Ceras/efectos adversos , Xerodermia Pigmentosa/genética , Xerodermia Pigmentosa/terapia , Zinc/deficienciaAsunto(s)
Humanos , Masculino , Femenino , Niño , Adolescente , Adulto Joven , Adulto , Enfermedad de Fabry/complicaciones , Enfermedad de Fabry/diagnóstico , Enfermedad de Fox-Fordyce/complicaciones , Enfermedad de Fox-Fordyce/diagnóstico , Enfermedad de Fox-Fordyce/terapia , Enfermedad de Fabry/fisiopatología , Enfermedad de Fabry/terapia , Enfermedad de Gaucher/complicacionesAsunto(s)
Enfermedad de Fox-Fordyce/diagnóstico , Inmunoglobulina G/metabolismo , Lactoglobulinas/inmunología , Xantomatosis/diagnóstico , Adulto , Axila , Carmín/química , Colorantes/química , Femenino , Células Espumosas/metabolismo , Células Espumosas/patología , Enfermedad de Fox-Fordyce/complicaciones , Enfermedad de Fox-Fordyce/metabolismo , Humanos , Mucinas/análisis , Mucinas/metabolismo , Coloración y Etiquetado , Xantomatosis/etiología , Xantomatosis/metabolismoAsunto(s)
Enfermedades en Gemelos/diagnóstico , Enfermedad de Fox-Fordyce/diagnóstico , Gemelos Monocigóticos , Axila , Biopsia con Aguja , Enfermedad Crónica , Enfermedades en Gemelos/tratamiento farmacológico , Enfermedades en Gemelos/patología , Femenino , Estudios de Seguimiento , Enfermedad de Fox-Fordyce/tratamiento farmacológico , Enfermedad de Fox-Fordyce/patología , Humanos , Inmunohistoquímica , Monitoreo Fisiológico/métodos , Enfermedades Raras , Adulto JovenRESUMEN
Fox-Fordyce disease is an uncommon disorder primarily affecting postpubertal females. It is characterized by intensely pruritic, papular eruptions in apocrine-gland bearing regions. Rarity and scant literature have resulted in a lack of definitive treatment options or pathognomonic diagnostic indicators. We report a 16-year-old boy with typical findings of Fox-Fordyce disease including numerous, grouped, rounded (1-3 mm), skin-colored papules confined to the axillary and periareolar areas, bilaterally. These lesions started before puberty, and were exacerbated by hyperhidrosis.
Asunto(s)
Axila , Enfermedad de Fox-Fordyce/diagnóstico , Hiperhidrosis/etiología , Adolescente , Hidróxido de Aluminio/uso terapéutico , Femenino , Enfermedad de Fox-Fordyce/complicaciones , Enfermedad de Fox-Fordyce/tratamiento farmacológico , Enfermedad de Fox-Fordyce/patología , Humanos , MasculinoRESUMEN
Fox-Fordyce disease (FFD) is a rare skin disease manifesting as multiple pruritic follicular papules involving the skin-bearing apocrine glands. Reports of FFD in Asian people are scant. In this retrospective study, we describe the clinicopathological findings of five cases of FFD affecting Taiwanese subjects. Clinically, all patients presented with numerous uniform, 2-3-mm, skin-colored to light brown, dome-shaped papules with smooth surface, which were distributed in the apocrine gland-containing areas. Pruritus varied from mild to severe. The histopathology is characterized by focal spongiosis in the upper infundibulum with perifollicular fibrosis and lymphohistiocytic infiltrate. FFD needs to be differentiated from lichen amyloidosis, Darier's disease, syringoma, lichen simplex chronicus and spongiotic dermatitis clinically or pathologically. The findings of focal spongiosis in upper infundibulum associated with a perifollicular lymphohistiocytic infiltrate can facilitate the diagnosis of FFD.
Asunto(s)
Enfermedad de Fox-Fordyce/patología , Adolescente , Axila/patología , Diagnóstico Diferencial , Femenino , Enfermedad de Fox-Fordyce/diagnóstico , Histiocitos/patología , Humanos , Queratosis/patología , Masculino , Adulto JovenRESUMEN
La enfermedad de Fox-Fordyce es una rara dermatosis caracterizada por la presencia de múltiples pápulas foliculares pruriginosas en áreas corporales con riqueza de glándulas apocrinas como axilas, areolas mamarias o región genital. Los hallazgos histopatológicos que definen la enfermedad de Fox-Fordyce son muy variados. Además de los hallazgos descritos como típicos de esta entidad, como la dilatación del infundíbulo y la hiperqueratosis y espongiosis del epitelio infundibular, se pueden observar otros muchos hallazgos histológicos. Presentamos el caso de una mujer de 21 años de edad afectada por esta enfermedad y recalcamos la importancia de la xantomatosis perinfundibular como hallazgo histológico clave en el diagnóstico de esta entidad (AU)
Fox-Fordyce disease is a rare skin condition characterized by the presence of multiple pruritic follicular papules in areas rich in apocrine glands, such as the axillae, mammary areolae, or genital regions. There is a high degree of variability in the histological findings seen in Fox-Fordyce disease. In addition to those described as typical of this entity, such as dilation of the infundibulum and hyperkeratosis and spongiosis of the infundibular epithelium, many other histological changes can be observed. We report the case of a 21-year-old woman with Fox-Fordyce disease and highlight the importance of perifollicular xanthomatosis as a key histological finding in the diagnosis of the disease (AU)